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首页> 外文期刊>North American Journal of Medical Sciences >Wilson's disease masquerading as nonalcoholic steatohepatitis
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Wilson's disease masquerading as nonalcoholic steatohepatitis

机译：威尔逊氏病伪装成非酒精性脂肪性肝炎

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Background:Wilson's disease is one of the most common hereditary causes of unclear hepatopathy.Patient & Method:A 34-year old male patient with a history of hyperlipidemia was admitted with symptoms of abdominal pain and slight hematuria. Abnormal liver function tests, ultrasound reports and liver biopsy were suggestive of nonalcoholic steatohepatitis (NASH). The patient received preliminary treatment for NASH. However, on subsequent follow-up, NASH remained unresolved and liver histology showed fibrosis progression from fibrosis stage 1 to stage 3.Results:Biochemical tests revealed that the levels of serum ceruloplasmin were decreased (7mg/dl) while the urinary excretion of copper was found to be increased (174.2 μg/day). Wilson's disease was confirmed by diagnostic mutation analysis involving Direct Sequencing. Heterogeneity in the patient's ATP7B gene confirmed Wilson's disease. Administration of D-penicillamine resulted in a decrease in fat deposition in the liver and no further progression in fibrosis after 10 months.Conclusion:Adult patient presenting NASH as first symptoms need to be examined for Wilson's disease and other metabolic conditions affecting the liver, prior to initiation of treatment.
机译：背景：威尔逊氏病是导致不清楚的肝病的最常见的遗传原因之一。患者与方法：一名34岁的高脂血症男性患者因腹部疼痛和轻微血尿症状而入院。肝功能检查异常，超声检查报告和肝活检提示非酒精性脂肪性肝炎（NASH）。该患者接受了NASH的初步治疗。然而，在随后的随访中，NASH仍未解决，肝组织学显示从纤维化第1阶段到第3阶段有纤维化进展。结果：生化测试显示，血清铜蓝蛋白水平降低了（7mg / dl），而尿中铜的排泄量却降低了。被发现增加（174.2微克/天）。威尔逊氏病已通过涉及直接测序的诊断突变分析得到证实。患者的ATP7B基因异质性证实了威尔逊氏病。服用D-青霉胺可导致10个月后肝脏脂肪沉积减少，且纤维化没有进一步进展。结论：以NASH为首发症状的成年患者需要检查威尔逊氏病和其他影响肝脏的代谢状况开始治疗。

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